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Anti-HGD Rabbit Polyclonal Antibody (ALEXA FLUOR® 750)

Description: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyze the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterized by urine that turns dark on standing and alkalinization, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Catalog Number: 76100-000

Supplier: Bioss

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Anti-Fumarase/FH Goat Polyclonal Antibody

Description: This antibody is predicted to react with canine, mouse and rat based on sequence homology. The protein encoded by this gene is an enzymatic component of the tricarboxylic acid cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.

Catalog Number: CAPIPA5-18745

Supplier: Thermo Scientific

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Anti-SDHB Rabbit Polyclonal Antibody

Description: SDHB(Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial) is also named as SDH, SDH1 and belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family. The SDHD, SDHB, and SDHC genes encode subunits of mitochondrial complex II (succinate dehydrogenase).Mitochondrial complex II is a heterotetrameric complex involved in the aerobic electron transport chain and catalyses the oxidation of succinate to fumarate (Krebs cycle) with transport of electrons to the ubiquinone pool. SDHA and SDHB are the hydrophilic catalytic part of the complex and are highly conserved(J Med Genet 2004;41:e99). Defects in SDHB are a cause of susceptibility to pheochromocytoma (PCC), paragangliomas type 4 (PGL4), paraganglioma and gastric stromal sarcoma (PGGSS) and Cowden-like syndrome (CWDLS).

Catalog Number: 10094-234

Supplier: Proteintech

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Dipentyl phthalate ≥98.0%

Description: CAS Number: 131-18-0
MDL Number: MFCD00041934
Molecular Formula: C18H26O4
Molecular Weight: 306.40
Purity/Analysis Method: >98.0% (GC)
Form: Clear Liquid
Boiling point (°C): 342
Flash Point (°C): 118
Specific Gravity (20/20): 1.03

Catalog Number: TCP0291-100G

Supplier: TCI America

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2-(2-Ethoxyethoxy)ethyl acrylate ≥98.0% stabilized

Description: (stabilized with MEHQ)
CAS Number: 7328-17-8
Molecular Formula: C9H16O4
Molecular Weight: 188.22
Purity/Analysis Method: >98.0% (GC)
Form: Clear Liquid
Boiling point (°C): 250
Flash Point (°C): 116
Specific Gravity (20/20): 1.02

Catalog Number: TCE0652-500G

Supplier: TCI America

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Human Recombinant 4HPPD (from E. coli)

Description: 4-Hydroxyphenylpyruvate Dioxygenase (4HPPD) belongs to the 4HPPD family. 4HPPD is a key enzyme in the degradation of tyrosine, which catalyzes the second reaction in the catabolism of tyrosine the conversation of 4-hydroxyphenylpyruvate to homogentisate. 4HPPD exists in homodimer forms, which uses zinc as a cofactor to catalyze the third step in the conversion of L-phenylalanine to fumarate and acetoacetic acid. When the active 4HPPD enzyme concentration is low in the human body, it results in high levels of tyrosine concentration in the blood, which can cause mild mental retardation at birth, and degradation in vision as a patient grows older.

Catalog Number: 75790-626

Supplier: Prosci

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Di-tert-butyl malonate ≥98.0%

Description: CAS Number: 541-16-2
MDL Number: MFCD00008810
Molecular Formula: C11H20O4
Molecular Weight: 216.28
Purity/Analysis Method: >98.0% (GC)
Form: Clear Liquid
Boiling point (°C): 67
Melting point (°C): -6
Flash Point (°C): 88
Specific Gravity (20/20): 0.97

Catalog Number: TCM1522-25G

Supplier: TCI America

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Anti-FAH Rabbit Polyclonal Antibody

Description: FAH is also named as FAA(fumarylacetoacetase), the last enzyme in tyrosine degradation, functionally important in the liver and kidney. FAH is a metabolic enzyme catalyzing the last step of tyrosine and phenylalanine catabolism: the hydrolysis of fumarylacetoacetate into acetoacetate and fumarate. In humans, deficiency of this activity is associated with the metabolic disease hereditary tyrosinaemia type I, which is also known as hepatorenal tyrosinaemia.

Catalog Number: 10086-770

Supplier: Proteintech

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4-Nitrophenyl phosphate disodium salt hexahydrate (pNPP)

Description: (pNPP, sodium). Pale yellow solid. Contaminants: free p-nitrophenol: <0.1%. CAS 4264-83-9, M.W. 263.1.

Catalog Number: CA80057-148

Supplier: MilliporeSigma

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DSS (Disuccinimidyl suberate) ≥98.0% (by HPLC, total nitrogen)

Description: CAS Number: 68528-80-3
MDL Number: MFCD00049059
Molecular Formula: C16H20N2O8
Molecular Weight: 368.34
Purity/Analysis Method: >98.0% (HPLC,N)
Form: Crystal
Melting point (°C): 171

Catalog Number: TCD3895-1G

Supplier: TCI America

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Anti-ADSL Rabbit Polyclonal Antibody

Description: ADSL(adenylosuccinate lyase) is also named as AMPS, ASase, ASL and belongs to the lyase 1 family. It is an enzyme involved in 2 pathways of purine nucleotide metabolism and catalyzes cleavage of succinyl groups to yield fumarate. Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). In humans, mutations in ADSL lead to an inborn error of metabolism originally characterized by developmental delay, often with autistic features..The ADSL enzymatic activity is reduced in lymphocytes and red blood cells of the patient with severe psychomotor retardation. It has 2 isoforms produced by alternative splicing.

Catalog Number: 10082-556

Supplier: Proteintech

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p-Nitrophenyl phosphate DiTris salt ≥90.0% (by titrimetric analysis)

Description: [Substrate for Phosphatase]
CAS Number: 68189-42-4
MDL Number: MFCD00037009
Molecular Formula: C6H6NO6P
Molecular Weight: 461.36
Purity/Analysis Method: >90.0% (T)
Form: Crystal
Storage Temperature: <0°C

Catalog Number: TCN0422-005G

Supplier: TCI America

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